Bomsztyk, JA; Pinney, JH; Lachmann, HJ; (2023) Amyloidosis and the lungs and airways. In: Wagner, Thomas OF and Humbert, Marc and Wijsenbeek, Marlies and Kreuter, Michael and Hebestreit, Helge, (eds.) Rare Diseases of the Respiratory System. (pp. 53-68). European Respiratory Society: Lausanne, Suisse.

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Abstract

Amyloidosis can both complicate long-standing respiratory conditions and be deposited within the respiratory system itself. In acquired systemic amyloidosis, control of the underlying condition that is producing the circulating amyloid precursor protein is paramount. Systemic AA amyloidosis can result from unremitting chronic inflammation or infection such as in bronchiectasis. Control of the inflammation is paramount to amyloid regression. For systemic AL amyloidosis, treatment requires the use of chemotherapy or novel immunotherapies targeting the underlying plasma cell dyscrasia or lymphoproliferative disease that produce the abnormal amyloidogenic light chain. Localised amyloidosis can occur anywhere along the respiratory tract and can present with marked heterogeneity. In localised amyloidosis, management generally involves resection or ablation of symptomatic deposits. On occasion, localised pulmonary amyloidosis can be a manifestation of underlying Sjögren syndrome. Novel treatments are beginning to become available, including specific drug therapies to prevent translation of amyloidogenic proteins, stabilise amyloid precursor proteins and interfere with amyloid fibrillogenesis.

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